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首页> 外文期刊>Endocrinology, Diabetes & Metabolism >Re-evaluating the prevalence and factors characteristic of catecholamine secreting head and neck paragangliomas
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Re-evaluating the prevalence and factors characteristic of catecholamine secreting head and neck paragangliomas

机译：重新评估儿茶酚胺分泌头和颈部的患病率和因子特征

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Introduction We sought to characterize the prevalence and factors characteristic of head and neck paragangliomas (HNPGLs) that secrete catecholamines to inform best practices for diagnosis and management. Methods This was a retrospective cohort study from 2000 to 2020 at a single-institution tertiary centre. One-hundred fifty-two patients (182 tumours) with HNPGLs with at least one measurement of urine or plasma catecholamines and/or catecholamine metabolite levels prior to treatment were included. We differentiated and characterized those patients with increased level(s) of any nature and those with ‘clinically significant’ versus ‘clinically insignificant’ catecholamine production. Results Thirty-one (20.4%) patients had increased catecholamine and/or catecholamine metabolite levels. In most patients, these levels were ≤5-fold above the upper limit of the reference range. Four of these 31 patients with increased levels were ultimately found to have an additional catecholamine secreting mediastinal paraganglioma or pheochromocytoma. Fourteen of 31 patients with HNPGL were deemed clinically significant secretors of catecholamines based on hyper-adrenergic symptoms and/or profound levels of normetanephrines. This cohort was enriched for patients with paragangliomas of the carotid body or cervical sympathetic chain and those with SDHB genetic mutations. Ultimately, the prevalence of clinically significant catecholamine secreting Hangs was determined to be 9.2% and 7.7% based on a per-patient and per-tumour basis, respectively. Conclusions The rate of catecholamine excess in the current cohort of patients with HNPGLs was higher than previously reported. Neuroendocrine tumours of any anatomic subsite may secrete catecholamines, although not all increased laboratory level(s) are indicative of clinically significant catecholamine secretion causing symptoms or warranting adrenergic blockade.
机译：引言我们寻求表征头部和颈部的患病率和因子特征，其分泌儿茶胺，以告知诊断和管理的最佳实践。方法这是一个从2000年到2020年的回顾性队列研究，在一个机构三级中心。包括含有至少一种测量的HNPGLs的一百五十二次患者（182颗肿瘤）在治疗前至少有一种测量尿液或血浆儿茶酚胺和/或儿茶酚胺代谢物水平。我们对这些患者进行了差异化，并表征了任何自然水平和具有“临床显着”与“临床上微不足道”的儿茶酚胺生产的患者。结果三十一（20.4％）患者增加了儿茶酚胺和/或儿茶酚胺代谢物水平。在大多数患者中，这些水平≤5倍高于参考范围的上限。最终发现这31例患者中的四种患者中有4例具有额外的儿茶酚胺分泌纵隔伞形脑膜瘤或嗜铬细胞瘤。基于高肾上腺素能症状和/或正常水平的常规癌症患者，31例HNPG1患者被视为临床上有临床的儿茶酚胺秘密。这种队列为颈动脉患者或颈椎同情链和具有SDHB遗传突变的患者而富集的患者。最终，基于每患者和每肿瘤的基础，确定临床显着的儿茶酚胺分泌悬挂的患病率分别为9.2％和7.7％。结论目前的HNPGL患者的儿茶酚胺过量率高于先前报道。任何解剖底座的神经内分泌肿瘤可能分泌儿茶酚胺，虽然并非所有的实验室水平都不是临床上显着的儿茶酚胺分泌，导致症状或保证肾上腺素能障碍。

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